Congenital occipitoatlantoaxial malformations in the horse

From a clinical, radiological and morphological study of 9 horses with congenital malformations of the occiput, atlas and axis, and from a study of 2 reported cases, 3 diseases were defined: A. Familial occipitalisation of the atlas with atlantalisation of the axis in Arabian horses (7 cases in this report and the case reported by Leipold, et al., 1974). These horses had congenital atlantooccipital fusion, hypoplasia of the atlas and dens, malformation of the axis and modification of the atlantoaxial joint. B. Congenital asymmetrical occipitoatlantoaxial malformation (2 cases in this report). A Standardbred and a Morgan horse had atlantooccipital fusion, a wedge shaped vertebral piece attached to the caudal end of the axis and sigmoid scoliosis of the cervical vertebrae. C. Asymmetrical atlantooccipital fusion (the case reported by Schmaltz, 1915). This horse of an unknown breed had asymmetrical fusion between the atlas and occiput and cervical scoliosis. The clinical syndromes shown by horses with these malformations were variable but were broadly classified as: 1. Foal dead at birth, seen in one foal with A. 2. Tetraparesis at birth, seen in 5 foals with A. These foals were born with signs varying from tetraparesis to tetraplegia. 3. Progressive ataxia, seen in 2 foals with A. Clinical signs were due to a progressive focal cervical compressive myelopathy. 4. Congenital cervical scoliosis/deviated head, seen in the 2 horses with B and the horse with C. These horses had no signs of spinal cord or brain disease. The diagnoses were made clinically by palpation of the occipitoatlantoaxial region and were confirmed radiographically and/or by post mortem examination in all except one case. Pedigree analysis showed the familial nature of the particular occipitoatlantoaxial malformation seen in horses of only the Arabian breed.     

Vertebral malformation, syringomyelia, and ventricular septal defect in a dromedary camel (Camelius dromedarius).

An occipitoatlantoaxial malformation and ventricular septal defect (VSD) were diagnosed in a 36-hour-old female camel. Physical examination revealed a firm protrusion of the dorsal aspect of the atlas and axis, tilting of the head to the left, and a grade V/VI systolic murmur. Neurological examination revealed proprioceptive deficits and ataxia of all 4 limbs. Radiographic examination and necropsy demonstrated malformation, fusion of the atlas to the occiput and hypoplasia of the dens of the axis, and subluxation of the atlantoaxial joint. Dorsoventral laxity of the atlantoaxial joint was also present, with compression of the cervical spinal cord. A 1.5-cm-diameter VSD was observed also. Histopathologic examination of the cervical spinal cord revealed a cavity extending from the level of the first to fourth cervical segment, dorsal to the central canal, 5 cm long and 1-2 mm in diameter. The cells around the cavity were positive for glial fibrillary acidic protein and sporadically positive for vimentin. This cavitary structure was consistent with syringomyelia, which was lined by glial cells, surrounded by edematous white matter with Wallerian-like degeneration and with neuronal necrosis in the adjacent dorsal horns.     

RADIOGRAPHIC AND MYELOGRAPHIC EXAMINATION OF THE CERVICAL VERTEBRAL COLUMN IN 306 ATAXIC HORSES

This study consists of a review of 306 ataxic horses subjected to survey radiography and myelography of the cervical vertebral column. Fifty-eight percent of horses had evidence of compressive spinal cord lesion on myelography. Sixty-six percent of horses were less than 3 years old. Age had no effect on the distribution of spinal cord compressive lesions. No conclusion could be drawn from sex and breed distribution. The most common sites of cord compression on myelography in order of decreasing frequency were C_3–4, C_6–7, C_5–6, and C_4–5. Twenty-nine percent of horses with myelographic evidence of cord compression had multiple compressive sites. Survey radiographs were not reliable to diagnose compressive spinal cord lesions. Myelography was a safe and more accurate procedure. Several factors may result in misinterpretation of the myelographic examination.     

Occipitoatlantoaxial malformation in two non-Arabian horses

Occipitoatlantoaxial malformation was diagnosed in a 19-month-old mare of Appaloosa breeding and in a 3-year-old Quarter Horse gelding. Both horses had abnormal head and neck carriage since birth, but neurologic deficits did not become evident clinically until the horses reached 2 and 3 years of age, respectively. Palpation and manipulation of the base of the skull and cervical area proved to be useful diagnostically. Movement could not be elicited at the atlantooccipital joint but, in comparison with clinically normal horses, the range of dorsoventral motion at the atlantoaxial joint was increased. Alternate subluxation and relocation of this joint generated audible clicking sounds. Radiography revealed symmetric atlantooccipital fusion, with modification of the atlas, atlantoaxial joint, and axis. These findings were confirmed at necropsy.     

Atlantoaxial subluxation and absence of transverse ligament of the atlas in a dog

Absence of the transverse ligament of the atlas was diagnosed at necropsy in an 8-month-old Shih Tzu with radiographic signs of atlantoaxial subluxation. Symmetric ataxia, tetraparesis, and signs of pain in the vertebral canal suggested a lesion in the cervical portion of the spinal cord. Necropsy revealed absence of the transverse ligament of the atlas and malformation of dens and atlas. In addition, the alar ligaments were distinct and thick, and the atlanto-occipital and atlantoaxial joint capsules were markedly thicker than normal. Histologic examination revealed focal compressive myelopathy of the spinal cord at the level of the atlantoaxial joint.     

Atlantoaxial cartilaginous exostosis causing spinal cord compression in a mature Bernese mountain dog

Cartilaginous exostosis developed in the atlantoaxial region of a three-and-a-half-year-old Bernese mountain dog. The dog exhibited ataxia in the hindlimbs and flailing movements in the forelimbs. On survey radiographs of the cervical spine there was a focal calcified mass between the dorsal arch of the atlas and the spinous process of the axis. Lumbar myelography revealed severe dorsal spinal cord compression. The mass was removed surgically and the dog made a complete recovery. Histopathology of the excised mass was consistent with a diagnosis of cartilaginous exostosis.     

Acute non-ambulatory tetraparesis in a 6-month-old Standardbred weanling caused by a cervical vertebral epidural haematoma

A 6-month-old Standardbred weanling presented with acute non-ambulatory tetraparesis. Cranial nerve examination was normal and neuroanatomic localisation suggested there was a focal C1-C5 spinal cord lesion. Post-mortem examination identified a cervical vertebral epidural haematoma at the level of C2-C3 causing spinal cord compression and neurological deficits. Histological examination determined the haematoma was several weeks old making the lesion chronic. Since the clinical progression was acute, this suggests an acute on chronic pathophysiology. Even with no history of trauma, an epidural haematoma should be on the differential list in young horses with acute tetraparesis.     

Atlanto-axial approach for cervical myelography in a Thoroughbred horse with complete fusion of the atlanto-occipital bones

A 2-year-old Thoroughbred gelding with clinical signs localized to the first 6 spinal cord segments (C1 to C6) had complete fusion of the atlanto-occipital bones which precluded performing a routine myelogram. An ultrasound-assisted myelogram at the intervertebral space between the atlas and axis was successfully done and identified a marked extradural compressive myelopathy at the level of the atlas and axis, and axis and third cervical vertebrae.     

Spinal compression due to atlantal vertebral malformation in two African lions (Panthera leo).

Two young (14-mo-old and 6-mo-old), unrelated, male African lions (Panthera leo) were presented to the Veterinary Teaching Hospitals of Oklahoma State University and Kansas State University with progressive ambulatory difficulty. In both cases, limited neurologic evaluation demonstrated pelvic limb paresis and ataxia with conscious proprioceptive deficits. Spinal imaging showed nearly identical lesions in both cases. Radiography and myelography demonstrated cervical stenosis secondary to atlantal (C1) malformation producing a dorsoventral deformity of the laminar arch with atlantoaxial spinal cord compression between the ventrally displaced laminar arch of the atlas and the underlying odontoid process of C2. Computed tomography of the atlanto-axial junction confirmed cervical stenosis and cord compression, showing flattening of the spinal cord between the laminar arch of C1 and the dens of C2. Decompressive surgery consisting of dorsal laminectomy of C1 was performed. Each lion demonstrated progressive improvement of neurologic status to recovery of normal ambulation after surgical intervention. Neurologic disease in large captive felids is rare; atlanto-axial spondylomyelopathy has not been reported previously.     

THE USE OF MAGNETIC RESONANCE IMAGING IN EVALUATING HORSES WITH SPINAL ATAXIA

To determine the accuracy of magnetic resonance imaging for diagnosing cervical stenotic myelopathy in horses, 39 horses with spinal ataxia and 20 control horses underwent clinical and neurologic examinations, cervical radiographs, euthanasia, magnetic resonance (MR) imaging of the cervical spine and necropsy. Twenty‐four horses were diagnosed with cervical stenotic myelopathy, 5 with cervical vertebral stenosis, 7 with idiopathic ataxia, 3 horses had other causes of ataxia, and 20 were controls. The MR images were assessed for spinal cord intensity changes, presence of spinal cord compression, spinal cord compression direction, shape of spinal cord, and the presence of synovial cysts, joint mice, and degenerative joint disease. The height, width, and area of the spinal cord, dural tube and vertebral canal were measured. The identification of spinal cord compression on MR images was significantly different in horses with cervical stenotic myelopathy (P < 0.02), but in the cervical stenotic myelopathy group the identification of spinal cord compression on MR images had poor to slight agreement with histopathologic evidence of compression (κ = 0.05). Horses with cervical stenotic myelopathy were more likely to have a T2 hyperintensity in the spinal cord (P < 0.05). Horses with cervical stenotic myelopathy or cervical vertebral stenosis were more likely to have degenerative joint disease than control horses or horses with other or idiopathic ataxia.     

Laminotomy of the Axis for Surgical Access to the Cervical Spinal Cord A Case Report

A cranially hinged laminotomy of vertebra C2 was used to expose the cervical spinal cord of a dog with a meningioma in the region of the atlantoaxial articulation. By preserving the dorsal atlantoaxial ligament, the technique seemed to result in greater and more physiologic stability between the atlas and axis than dorsal laminectomy and prosthetic replacement of the dorsal atlantoaxial ligament. The procedure allowed a dorsal approach, avoiding injury to the vertebral arteries and limited exposure, which are potential problems with hemilaminectomy of C1-C2. Further investigation is needed to evaluate long-term consequences of this procedure.     

Surgical decompression for traumatic atlantoaxial subluxation in a weanling filly.

A filly with ataxia and splinting and crepitation in the neck was found to have atlantoaxial subluxation. Radiographic diagnosis was based on the same criteria as those used in other species, ie, increased distance between the atlas and spine of the axis and increased distance between the dens and floor of the atlas. Extensive hemilaminectomy was performed to decompress the spinal cord. Stabilization was not attempted. Immediate postoperative response was encouraging, but the untimely death of the filly prevented further evaluation of the procedure.     

Occipitoatlantoaxial malformation with duplication of the atlas and axis in a half Arabian foal

An unusual occipitoatlantoaxial malformation is described in a 2-week-old male part Arabian foal that was unable to stand at birth and showed signs of spastic tetraparesis due to a cervical spinal cord compression. There were 2 atlases present. One was fused to the occipital bones. The other articulated with the first atlas and an axis which had a long dens that projected into the vertebral canal. Examination of the ossification centers of the axis indicated partial duplication of that bone.     

Congenital occipitoatlantoaxial malformation in a Warmblood mare

A 3-year-old Warmblood mare was presented with spinal ataxia of 3 months’ duration. Clinically, reduced mobility of the neck and a prominent atlas were identified. Neurological evaluation revealed hypermetria, circumduction and reduced proprioception, predominantly in the hindlimbs. Occipitoatlantoaxial malformation (OAAM) was diagnosed based on radiography and computed tomography, and confirmed on post-mortem magnetic resonance imaging and pathological examination. Both the atlas and axis were hypoplastic and dysplastic, and a fusion of the atlanto-occipital joint was found. A dynamic compression of the spinal cord was present at the first and second cervical vertebrae. Histologically, the spinal cord was degenerated due to compression. Furthermore, there were muscular malformations including a fibrotic obliquus capitis cranialis muscle, aplasia of parts of the rectus capitis muscles and presence of additional abnormal muscle. Such muscular abnormalities associated with OAAM have not been reported yet and this is the first report of a case of OAAM in a Warmblood horse.     

Chronic cervical compressive myelopathy in horses: patterns of astrocytosis in the spinal cord

The distribution and morphology of fibrous astrocytes in the cervical spinal cord of normal horses and horses with chronic compressive myelopathy were demonstrated using immunohistochemical staining for glial fibrillary acidic protein. In the spinal cord from normal horses, astrocytes with stellate cell bodies and short processes were irregularly distributed in grey matter. In the white matter, their cell bodies were small and angular in areas adjacent to grey matter and larger and more stellate-shaped in the subpial area. Astrocyte processes were fine, and evenly distributed in a predominantly radial pattern in transverse sections of cord. Gliosis was marked in the spinal cords of horses with cervical compressive myelopathy. In the grey matter at the level of compression astrocytes were often enlarged and rounded, with short, blunt processes, but the gliosis was generally mild. In the white matter, gliosis was obvious in areas of nerve fibre swelling and degeneration at the level of compression and in areas of ascending and descending Wallerian degeneration. The fine radial pattern of astrocyte fibres was replaced by a dense, irregular arrangement. Gliosis persisted in the cords of chronically affected horses after active nerve fibre degeneration had subsided. The areas of gliosis coincided with the areas of Marchi staining for degenerating myelin and with areas of myelin loss in osmium tetroxide post-fixed tissue. Histological observations were consistent with astrocytes replacing areas of extracellular space that remained after nerve fibre degeneration. it is concluded that astrocytic gliosis is a prominent and persistent alteration of the spinal cord of horses with chronic cervical compressive myelopathy.     

Contrast-enhanced computed tomography and myelography in six horses with cervical stenotic myelopathy.

The cervical spines of 6 horses with cervical stenotic myelopathy (CSM) were examined using myelography and contrast-enhanced computed tomography (CECT). Histopathology of the spinal cord of these horses identified 10 neurologically significant compressive lesions. Myelography and CECT were both able to demonstrate all 10 spinal cord compressive lesions, but myelography falsely identified 2 sites and CECT falsely identified 1 site as compressive lesions of the spinal cord which were not supported by histopathology. Additional qualitative information was obtained by CECT regarding the source, severity and location of spinal cord compression. Computed tomography identified stenosis of the vertebral canal with circumferential loss of contrast agent and documented lateral compressive lesions of the spinal cord due to malformed articular facets. Compression of the peripheral nerve roots by malformed articular facets encroaching on the intervertebral foramen was easily identified by CECT in the axial plane. No compressive lesions were identified in 3 unaffected horses by either method. Minimum sagittal diameter (MSD) values obtained from CECT images were strongly correlated with necropsy measurements, validating CECT as an accurate method of obtaining MSD values. The MSD values in the CSM-affected horses were significantly narrowed (P less than 0.05) from C3C6 regardless of the site of spinal cord compression, when compared with the unaffected controls. This finding supports previous reports suggesting that generalised stenosis of the vertebral canal is an important feature in the pathogenesis of cervical stenotic myelopathy.     

Confirmed and presumptive cervical vertebral compressive myelopathy in older horses: a retrospective study (1992-2004)

BACKGROUND: Cervical vertebral compressive myelopathy (CVCM) is a common cause of myelopathy in horses aged 6 months to 4 years. Little information is available regarding the types of lesions, treatment, and outcomes in horses with CVCM that are > or =4 years old. ANIMALS: Twenty-two affected horses (10 with a confirmed diagnosis of CVCM and 12 presumptive cases) and 210 contemporaneous control horses. METHODS: Horses > or =4 years old that were diagnosed with CVCM between January 1992 and January 2004 were identified from medical records at Texas A&M University and the University of Florida. Data analyzed included history, signalment, neurologic examination findings, lesion location, treatment, and outcome. Signalment was also recorded in a population of contemporaneous controls. RESULTS: Horses identified had a median age of 8.4 years, and there was a greater percentage of male horses among the cases than among the controls. The most common breeds represented were warmblood (n=6) and quarter horse (n=5) types; warmbloods were significantly (P < .05) overrepresented relative to control horses. The caudal cervical vertebral column was the most common site of CVCM lesions, and the C5-C6 (4/9) and C6-C7 (3/9) articulations were most often identified as abnormal via myelography. The most common lesions seen with radiography and myelography were articular process osteophytes. Of the 22 affected horses, 8 were euthanized and a diagnosis of CVCM was confirmed by necropsy for all; 5 of 8 of these horses had spinal cord compression caused, entirely or in part, by articular process osteophytes. Medical management was the therapy chosen in all horses, and administration of corticosteroids and nonsteroidal anti-inflammatory drugs resulted in improvement in the greatest number of horses. CONCLUSIONS AND CLINICAL IMPORTANCE: CVCM should be a differential diagnosis in older horses with cervical myelopathy. Articular process osteophytes are the most frequently identified cause of spinal cord compression in this group. Male horses and horses of warmblood or Tennessee Walking Horse breeds may be predisposed to this condition.     

Ataxia and weakness associated with fourth ventricle vascular anomalies in two horses

Two adult horses with progressive neurologic signs were examined clinically and at necropsy. Both horses had signs of progressive ataxia and weakness, clinically diagnosed as spinal cord in origin. Differential diagnoses for cervical spinal ataxia in horses included cervical vertebral malformation, equine degenerative myeloencephalopathy, equine herpes-virus-I myeloencephalopathy, and equine protozoal myeloencephalopathy. Necropsy findings in both horses were similar and consisted of a large hematoma in the fourth ventricle, with upward compression of the cerebellum and downward compression of the pons and rostral portion of the medulla.     

Spinal trauma. Pathophysiology and management of traumatic spinal injuries.

Spinal trauma can originate from internal or external sources. Injuries to the spinal cord can be classified as either concussive or compressive and concussive. The pathophysiologic events surrounding spinal cord injury include the primary injury (compression, concussion) and numerous secondary injury mechanisms (vascular, biochemical, electrolyte), which are mediated by excessive oxygen free radicles, neurotransmitter and electrolyte alterations in cell membrane permeability, excitotoxic amino acids, and various other biochemical factors that collectively result in reduced SCBF, ischemia, and eventual necrosis of the gray and white matter. Management of acute spinal cord injuries includes the use of a high-dose corticosteroid regimen within the initial 8 hours after trauma. Sodium prednisolone and methylprednisolone, at recommended doses, act as oxygen radical scavengers and are anti-inflammatory. Additional considerations are the stability of the vertebral column, other conditions associated with trauma (i.e., pneumothorax), and the presence or absence of spinal cord compression, which may warrant surgical therapy. Vertebral fractures or luxations can occur in any area of the spine but most commonly occur at the junction of mobile and immobile segments. Dorsal and dorsolateral surgical approaches are applicable to the lumbosacral and thoracolumbar spine and dorsal and ventral approaches to the cervical spine. Indications for surgical intervention include spinal cord compression and vertebral instability. Instability can be determined from the type of fracture, how many of the three compartments of the vertebrae are disrupted, and on occasion, by carefully positioned stress studies of fluoroscopy. Decompression (dorsal laminectomy, hemilaminectomy, or ventral cervical slot) is employed when compression of the spinal cord exists. The hemilaminectomy (unilateral or bilateral) causes less instability than dorsal laminectomy and therefore should be used when practical. The preferred approach for atlantoaxial subluxation is ventral, and the cross pinning, vertebral fusion technique is used for stabilization. Fracture luxations of C-2 are repaired with small plates on the ventral vertebral body. The thoracic and upper lumbar spine is stabilized with dorsal fixation techniques or combined dorsal spinal plate/vertebral body plate fixation. Several methods of fixation can be used with lower lumbar or lumbosacral fractures, including the modified segmental technique and the combined dorsal spinal plate/Kirschner-Ehmer technique.